The pattern of CD38 expression defines a distinct subset of chronic lymphocytic leukemia (CLL) patients at risk of disease progression
The pattern of CD38 expression defines a distinct subset of chronic lymphocytic leukemia (CLL) patients at risk of disease progression
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DOI：
10.1182/blood-2002-06-1801
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发表时间：
2003-02-15
期刊：
影响因子：
20.3
通讯作者：
Caligaris-Cappio, F
中科院分区：
文献类型：
--
作者：
Ghia, P;Guida, G;Caligaris-Cappio, F
慢性淋巴细胞白血病（CLL）有一个可变的临床过程。CD 38表达和IgV（H）基因突变状态是预后的独立预测因子，但它们之间的关系和CD 38临界水平尚不清楚。我们使用细胞荧光造影术分析了148例患者的CD 38，其中108例我们能够评估IgV（H）突变，与病史相关，并评估累积生存率。通过CD 38表达模式确定了三个不同的患者组：一组为均匀CD 38（-），一组为均匀CD 38（+），另一组为双峰型，因为同时存在不同比例的2个不同群体，一个CD 38（+）和一个CD 38（-）。在CD 38双峰表达的患者中，CD 38（+）亚群在骨髓中比在外周血中明显更多。对于IgV（H）突变，11.4%的CD 38-、84.6%的CD 38（+）和68.0%的CD 38双峰表达患者没有突变。CD 38表达、19V（H）突变状态和传统预后因素是一致的。CD 38-、CD 38（+）和CD 38双峰表达患者的进展率分别为12.9%、75.0%和63.3%。只有25.8%的CD 38（-）患者接受了治疗，但63.3%的双峰患者和75.0%的CD 38（+）患者接受了治疗。CD 38（+）人群的存在，尽管很小，但与自身免疫表现的发展相关。无论CD 38（+）群体的大小，CD 38（-）组均已达到中位生存期，其在CD 38（+）组中为183个月，在CD 38双峰表达组中为156个月。在白血病克隆中存在不同的CD 38（+）群体，而不是一个数值截止定义，与IgV（H）基因突变状态相关，并且无论其大小如何，都可以识别将具有进展性疾病的CLL患者。(C)2003年，美国血液学会。
Chronic lymphocytic leukemia (CLL) has a variable clinical course. CD38 expression and IgV(H) gene mutational status are independent predictors of prognosis, but their relationships and the CD38 cutoff level are unknown. Using cytofluorography, we analyzed CD38 in 148 patients, in 108 of whom we were able to evaluate IgV(H) Mutations, make correlations with disease history, and assess cumulative survival. Three different patient groups were identified by the CD38 expression pattern: a group homogeneously CD38(-), a group homogeneously CD38(+), and a group characterized by a bimodal profile, because of the concomitant presence of variable proportions of 2 distinct populations, one CD38(+) and one CD38(-). In CD38 bimodal expression patients the CD38(+) subset was significantly more represented in the bone marrow than in the peripheral blood. For IgV(H) mutations, 11.4% of CD38-, 84.6% of CD38(+), and 68.0% of CD38 bimodal expression patients had no mutation. CD38 expression, 19V(H) mutational status, and traditional prognostic factors were concordant. The progression rate was 12.9% for CD38-, 75.0% for CD38(+), and 63.3% for CD38 bimodal expression patients. Only 25.8% of the CD38(-) patients but 63.3% of the bimodal and 75.0% of CD38(+) patients were treated The presence of a CD38(+) population, albeit small, correlated with the development of auioimmune manifestations. T I he CD38(-) group has hot yet reached the median survival, which is 183 months in the CD38(+) group and 156 months in the CD38 bimodal expression group, regardless of the size of the CD38(+) population. The presence of a distinct CD38(+) population within the leukemic clone, rather than a numerical cutoff definition, correlates with IgV(H) gene mutational status and, irrespective of its size, identifies CLL patients who will have progressive disease. (C) 2003 by The American Society of Hematology.